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*MHD Exam 5 > Cystic Lesions and Tumors > Flashcards

Cystic Lesions and Tumors Flashcards

1
Q

What is the best way to differentiate renal cystic diseases?

A
  • age
  • incidence
  • complications
  • reniform vs. irregular
  • bilateral vs. unilateral vs. segmental
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2
Q

What are simple cysts?

A

very common, usually asymptomatic cysts that we only worry about when they become complex and symptomatic (hemorrhage, pain, calcifications) - look into potential cystic cancer

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3
Q

Acquired cystic disease is caused by:

A

dialysis

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4
Q

Patients with dialysis-associated cystic disease are at an increased risk of developing what?

A

renal cell carcinoma

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5
Q

What are the medullary diseases with cysts?

A
  • Medullary sponge (common, innocuous)

- Nephronophtisis-medullary cystic disease complex (common cause of pediatric onset CKD)

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6
Q

What are the adult benign kidney tumors?

A
  • Oncocytoma

- Angiomyolipoma

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7
Q

What is the main childhood kidney tumor to know? Is it benign or malignant?

A

Wilms tumor

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8
Q

What are the adult malignant tumors?

A

renal clear cell carcinoma, papillary renal cell carcinoma, chromophobe renal carcinoma, urothelial carcinoma of the renal pelvis/ureter

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9
Q

What is the characteristic gross appearance of an oncocytoma?

A

mahogany brown with central scar

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10
Q

What is the triad associated with renal cell carcinoma (RCC)?

A

painless hematuria, palpable abdominal mass, dull flank pain

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11
Q

What is seen on histology and EM with oncocytoma?

A

eosinophilic cytoplasm and abundant mitochondria on EM

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12
Q

Renal Cell Carcinoma is notoriously associated with which structure?

A

inferior vena cava

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13
Q

What are the risk factors for developing RCC?

A

tobacco use, obesity, HTN, occupational cadmium exposure, acquired cystic disease (30-fold increased risk)

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14
Q

Angiomyolipoma is often associated with which condition?

A

Tuberous Sclerosis

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15
Q

Angiomyolipomas usually appear in which tissues?

A

vessels, smooth muscle, and fat

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16
Q

Even though amgiomyolipomas are benign, what can happen that makes them dangerous?

A

large ones can rupture and cause bleeding

17
Q

Which syndrome is associated with RCC clear cell type?

A

von Hippel-Lindau (VHL)

18
Q

Are most RCCs sporadic or familial?

A

sporadic (only familial 4% of cases)

19
Q

Which mutation is implicated in clear cell renal cell carcinoma?

A

somatic mutation/hypermethylation/induced inactivation of the VHL gene (a tumor suppressor)

20
Q

Which part of the nephron can give rise to tumor?

A

all segments, except for the glomerulus

21
Q

What are the first and second most common RCCs?

A
  1. clear cell

2. papillary

22
Q

What does renal cell carcinoma look like on histology?

A

cells w/ clear cytoplasm; highly vascular; delicate, “chicken wire”-like blood vessels

23
Q

Papillary renal cell carcinoma is associated with which gene?

A

MET oncogene

24
Q

This type of RCC has morphologic overlap with oncocytoma.

A

Chromophobe Renal Cell Carcinoma (“vegetable-like” cells)

25
Q

What is the prognosis for the different types of RCC from best to worst?

A

chromophobe > papillary > clear cell

26
Q

What is the treatment for RCC?

A
  • surgery=mainstay
  • immunotherapy
  • targeted therapies (becoming more common)
  • NOT chemo (RCC is very chemo-resistant)
27
Q

mTOR activation promotes:

A
  • cell growth/proliferation
  • angiogenesis
  • cancer cell metabolism through increased nutrient uptake/utilization
28
Q

Which type of cancer affects the renal pelvis and ureters?

A

urothelial carcinoma

29
Q

What is the classic clinical presentation of urothelial carcinoma?

A

hematuria

30
Q

Wilms tumor is associated with which syndromes?

A
  • Beckwith-Wiedemann syndrome
  • WAGR complex
  • Denys-Drash syndrome
31
Q

What mutation is implicated in Wilms tumor?

A

WT1 or WT2 (loss of function)

32
Q

What are the components of Wilms tumor triphasic histology?

A
  • undifferentiated blastema
  • tubules
  • fibroblast-like stroma
33
Q

_________ are the putative precursor lesions of Wilms tumor.

A

Nephrogenic rests

34
Q

Wilms tumors are caused by a combination of which abnormal processes?

A
  • teratogenesis (abnormal development)

- oncogenesis (tumor induction)

35
Q

Wilms tumors recapitulate _________.

A

nephrogenesis

36
Q

What is the treatment for Wilms tumor?

A
  • surgery with OR without chemotherapy, depending on stage

* Wilms tumors ARE chemosensitive!

37
Q

What is the most common pediatric malignancy from 0-4 years of age?

A

neuroblastoma (adrenal gland) and Wilms tumor (kidney)

*MHD Exam 5 (14 decks)

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